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[阴囊多发性福代斯样血管角化瘤。一例医源性病例]

[Multiple Fordyce-type angiokeratomas of the scrotum. An iatrogenic case].

作者信息

Bisceglia M, Carosi I, Castelvetere M, Murgo R

机构信息

Servizio di Anatomia Patologica, IRCCS-Ospedale, Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG).

出版信息

Pathologica. 1998 Feb;90(1):46-50.

PMID:9628980
Abstract

BACKGROUND

Angiokeratoma is a wart-like vascular lesion of the skin. There are five types of angiokeratoma: the Mibelli-type, the Fordyce-type, the solitary and multiple (papular) types, the angiokeratoma circumscriptum, and the angiokeratoma corporis diffusum. The "Mibelli-type" occurs on the acral sites, mainly digits, of young people affected by repeated attacks of chilblain, which result in a deleterious effects on vessel walls. The "Fordyce-type" occurs on the scrotal skin of young and adults as a secondary effect to an increased blood pressure in scrotal veins. An equivalent form affecting adult females and occurring analogously on the skin of the vulva is also on record. The "solitary and multiple papular types" of young individuals affect the lower extremities and is considered a consequence of a congenital deficiency of elastic tissue in regional veins. We suggest the term "acquired angiokeratomas" for these three above mentioned clinical forms of angiokeratomas, leaving apart the other two types which are essentially congenital. In fact the "angiokeratoma circumscriptum" is a nevoid hamartomatous lesion arising early in life during infancy or childhood, sometimes in association with other congenital malformation of other sites, while the "angiokeratoma corporis diffusum" almost always occurs in association with enzyme disorders, usually alpha-galactosidase A enzyme deficiency (Anderson-Fabry disease), an X-linked recessive disorder affecting homozigous male patients in their adulthood.

CASE REPORT

A case of multiple angiokeratomas of the scrotum (Fordyce-type) arisen in a 62-year old male patient following surgical amputation of the penis and bilateral inguino-crural lymphadenectomy for carcinoma of the penis is reported on.

RESULTS AND CONCLUSION

Although they are well on record cases of angiokeratomas of the scrotum arising after surgical injuries to the outer vein pudenda (mainly following inguinocrural hernioplasty), based on a computerized search of the literature on theme this case represents the first iatrogenic example of such an occurrence. The pathogenetic mechanism leading to the the rise of angiokeratomas mediated by the increase of the blood pressure in the superficial scrotal veins is discussed. This example represents an additional case report of inevitable and sometimes unsuspected iatrogenic pathology in medicine.

摘要

背景

血管角化瘤是一种皮肤的疣状血管病变。血管角化瘤有五种类型:米贝利型、福代斯型、孤立性和多发性(丘疹型)、局限性血管角化瘤和弥漫性躯体血管角化瘤。“米贝利型”发生于受冻疮反复侵袭的年轻人的手足部位,主要是手指,冻疮会对血管壁造成有害影响。“福代斯型”发生于年轻人和成年人的阴囊皮肤,是阴囊静脉血压升高的继发效应。有一种类似的情况也有记录,发生于成年女性,出现在外阴皮肤。年轻人的“孤立性和多发性丘疹型”累及下肢,被认为是局部静脉弹性组织先天性缺乏的结果。我们建议将上述三种血管角化瘤的临床类型称为“后天性血管角化瘤”,而将另外两种本质上为先天性的类型排除在外。事实上,“局限性血管角化瘤”是一种痣样错构瘤性病变,在婴儿期或儿童期生命早期出现,有时与其他部位的其他先天性畸形有关,而“弥漫性躯体血管角化瘤”几乎总是与酶紊乱相关,通常是α-半乳糖苷酶A酶缺乏(安德森-法布里病),这是一种X连锁隐性疾病,成年男性纯合子患者会受其影响。

病例报告

报告了一例62岁男性患者,在因阴茎癌接受阴茎手术切除及双侧腹股沟-股淋巴结清扫术后出现阴囊多发性血管角化瘤(福代斯型)。

结果与结论

虽然有文献记载,在外阴部外静脉手术损伤后(主要是腹股沟-股疝修补术后)会出现阴囊血管角化瘤,但经计算机检索该主题文献,此病例是首例医源性阴囊血管角化瘤。本文讨论了阴囊浅表静脉血压升高介导血管角化瘤发生的发病机制。该病例是医学中不可避免且有时难以预料的医源性病理的又一病例报告。

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